U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Primary mediastinal large B-cell lymphoma

MedGen UID:
714984
Concept ID:
C1292754
Neoplastic Process
Synonyms: B-Cell Diffuse Large Cell Lymphoma of Mediastinum; B-cell diffuse large cell lymphoma of mediastinum; B-Cell Diffuse Large Cell Lymphoma of the Mediastinum; B-cell diffuse large cell lymphoma of the mediastinum; Large cell lymphoma of the mediastinum; large cell lymphoma of the mediastinum; Med-DLBCL; Mediastinal (thymic) large B-cell lymphoma; mediastinal (thymic) large B-cell lymphoma; Mediastinal B-Cell Diffuse Large Cell Lymphoma; mediastinal B-cell diffuse large cell lymphoma; mediastinal diffuse large cell lymphoma with sclerosis; Mediastinal diffuse large-cell lymphoma with sclerosis; mediastinal diffuse large-cell lymphoma with sclerosis; Mediastinal large B-cell lymphoma; mediastinal large B-cell lymphoma; PMBL; PMLCL; Primary mediastinal (thymic) large B-cell lymphoma; Primary Mediastinal (Thymic) Large B-Cell Lymphoma; primary mediastinal (thymic) large B-cell lymphoma; primary mediastinal B-cell lymphoma; primary mediastinal clear cell lymphoma of B-cell type; Primary mediastinal clear cell lymphoma of B-cell type; Primary Mediastinal Large B-Cell Lymphoma; primary mediastinal large B-cell lymphoma; Thymic large B-cell lymphoma
SNOMED CT: Thymic large B-cell lymphoma (128801005); Mediastinal large B-cell lymphoma (128801005); Mediastinal (thymic) large B-cell lymphoma (444910004); Primary mediastinal (thymic) large B-cell lymphoma (444910004)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0020323
Orphanet: ORPHA98838

Definition

A rare subtype of diffuse large B-cell lymphoma (DLBCL), arising from B cells of thymic origin, predominantly affecting women between the ages of 20-30, and that usually presents with a bulky and rapidly expanding anterior mediastinal mass, often with pleural and pericardial effusions, and that can invade the lungs, superior vena cava, pleura, pericardium, and chest wall, leading to manifestations of cough, dyspnea, and superior vena cava syndrome. [from ORDO]

Professional guidelines

PubMed

Ahmed Z, Afridi SS, Shahid Z, Zamani Z, Rehman S, Aiman W, Khan M, Mir MA, Awan FT, Anwer F, Iftikhar R
Clin Lymphoma Myeloma Leuk 2021 Nov;21(11):e865-e875. Epub 2021 Jun 24 doi: 10.1016/j.clml.2021.06.012. PMID: 34330673
Zhang ML, Sohani AR
Semin Diagn Pathol 2020 Jul;37(4):156-165. Epub 2020 May 15 doi: 10.1053/j.semdp.2020.04.005. PMID: 32451144
Martelli M, Di Rocco A, Russo E, Perrone S, Foà R
Expert Rev Hematol 2015 Apr;8(2):173-86. Epub 2014 Dec 24 doi: 10.1586/17474086.2015.994604. PMID: 25537750

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Aggressive Mature B-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Noerenberg D, Briest F, Hennch C, Yoshida K, Hablesreiter R, Takeuchi Y, Ueno H, Staiger AM, Ziepert M, Asmar F, Locher BN, Toth E, Weber T, Amini RM, Klapper W, Bouzani M, Poeschel V, Rosenwald A, Held G, Campo E, Ishaque N, Stamatopoulos K, Kanellis G, Anagnostopoulos I, Bullinger L, Goldschmidt N, Zinzani PL, Bödör C, Rosenquist R, Vassilakopoulos TP, Ott G, Ogawa S, Damm F
J Clin Oncol 2024 Feb 1;42(4):452-466. Epub 2023 Dec 6 doi: 10.1200/JCO.23.01053. PMID: 38055913
Armand P, Rodig S, Melnichenko V, Thieblemont C, Bouabdallah K, Tumyan G, Özcan M, Portino S, Fogliatto L, Caballero MD, Walewski J, Gulbas Z, Ribrag V, Christian B, Perini GF, Salles G, Svoboda J, Zain J, Patel S, Chen PH, Ligon AH, Ouyang J, Neuberg D, Redd R, Chatterjee A, Balakumaran A, Orlowski R, Shipp M, Zinzani PL
J Clin Oncol 2019 Dec 1;37(34):3291-3299. Epub 2019 Oct 14 doi: 10.1200/JCO.19.01389. PMID: 31609651Free PMC Article
Zinzani PL, Santoro A, Gritti G, Brice P, Barr PM, Kuruvilla J, Cunningham D, Kline J, Johnson NA, Mehta-Shah N, Manley T, Francis S, Sharma M, Moskowitz AJ
J Clin Oncol 2019 Nov 20;37(33):3081-3089. Epub 2019 Aug 9 doi: 10.1200/JCO.19.01492. PMID: 31398081Free PMC Article
Sandlund JT
Curr Hematol Malig Rep 2015 Sep;10(3):237-43. doi: 10.1007/s11899-015-0277-y. PMID: 26174528
Bhatt VR, Mourya R, Shrestha R, Armitage JO
Cancer Treat Rev 2015 Jun;41(6):476-85. Epub 2015 Apr 21 doi: 10.1016/j.ctrv.2015.04.006. PMID: 25913715

Diagnosis

Savage KJ
Blood 2022 Sep 1;140(9):955-970. doi: 10.1182/blood.2020008376. PMID: 34496020
Yu Y, Dong X, Tu M, Wang H
Thorac Cancer 2021 Nov;12(21):2831-2837. Epub 2021 Sep 29 doi: 10.1111/1759-7714.14155. PMID: 34590432Free PMC Article
Ahmed Z, Afridi SS, Shahid Z, Zamani Z, Rehman S, Aiman W, Khan M, Mir MA, Awan FT, Anwer F, Iftikhar R
Clin Lymphoma Myeloma Leuk 2021 Nov;21(11):e865-e875. Epub 2021 Jun 24 doi: 10.1016/j.clml.2021.06.012. PMID: 34330673
Xue Y, Gao S, Gou J, Yin T, He H, Wang Y, Zhang Y, Tang X, Wu R
Expert Opin Drug Deliv 2021 Feb;18(2):187-203. Epub 2020 Oct 5 doi: 10.1080/17425247.2021.1825376. PMID: 32954856
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345

Therapy

Zinzani PL, Thieblemont C, Melnichenko V, Bouabdallah K, Walewski J, Majlis A, Fogliatto L, Garcia-Sancho AM, Christian B, Gulbas Z, Özcan M, Perini GF, Ghesquieres H, Shipp MA, Thompson S, Chakraborty S, Marinello P, Armand P
Blood 2023 Jul 13;142(2):141-145. doi: 10.1182/blood.2022019340. PMID: 37130017Free PMC Article
Savage KJ
Blood 2022 Sep 1;140(9):955-970. doi: 10.1182/blood.2020008376. PMID: 34496020
Armand P, Rodig S, Melnichenko V, Thieblemont C, Bouabdallah K, Tumyan G, Özcan M, Portino S, Fogliatto L, Caballero MD, Walewski J, Gulbas Z, Ribrag V, Christian B, Perini GF, Salles G, Svoboda J, Zain J, Patel S, Chen PH, Ligon AH, Ouyang J, Neuberg D, Redd R, Chatterjee A, Balakumaran A, Orlowski R, Shipp M, Zinzani PL
J Clin Oncol 2019 Dec 1;37(34):3291-3299. Epub 2019 Oct 14 doi: 10.1200/JCO.19.01389. PMID: 31609651Free PMC Article
Zinzani PL, Santoro A, Gritti G, Brice P, Barr PM, Kuruvilla J, Cunningham D, Kline J, Johnson NA, Mehta-Shah N, Manley T, Francis S, Sharma M, Moskowitz AJ
J Clin Oncol 2019 Nov 20;37(33):3081-3089. Epub 2019 Aug 9 doi: 10.1200/JCO.19.01492. PMID: 31398081Free PMC Article
Xu-Monette ZY, Zhou J, Young KH
Blood 2018 Jan 4;131(1):68-83. Epub 2017 Nov 8 doi: 10.1182/blood-2017-07-740993. PMID: 29118007Free PMC Article

Prognosis

Noerenberg D, Briest F, Hennch C, Yoshida K, Hablesreiter R, Takeuchi Y, Ueno H, Staiger AM, Ziepert M, Asmar F, Locher BN, Toth E, Weber T, Amini RM, Klapper W, Bouzani M, Poeschel V, Rosenwald A, Held G, Campo E, Ishaque N, Stamatopoulos K, Kanellis G, Anagnostopoulos I, Bullinger L, Goldschmidt N, Zinzani PL, Bödör C, Rosenquist R, Vassilakopoulos TP, Ott G, Ogawa S, Damm F
J Clin Oncol 2024 Feb 1;42(4):452-466. Epub 2023 Dec 6 doi: 10.1200/JCO.23.01053. PMID: 38055913
Ahmed Z, Afridi SS, Shahid Z, Zamani Z, Rehman S, Aiman W, Khan M, Mir MA, Awan FT, Anwer F, Iftikhar R
Clin Lymphoma Myeloma Leuk 2021 Nov;21(11):e865-e875. Epub 2021 Jun 24 doi: 10.1016/j.clml.2021.06.012. PMID: 34330673
Armand P, Rodig S, Melnichenko V, Thieblemont C, Bouabdallah K, Tumyan G, Özcan M, Portino S, Fogliatto L, Caballero MD, Walewski J, Gulbas Z, Ribrag V, Christian B, Perini GF, Salles G, Svoboda J, Zain J, Patel S, Chen PH, Ligon AH, Ouyang J, Neuberg D, Redd R, Chatterjee A, Balakumaran A, Orlowski R, Shipp M, Zinzani PL
J Clin Oncol 2019 Dec 1;37(34):3291-3299. Epub 2019 Oct 14 doi: 10.1200/JCO.19.01389. PMID: 31609651Free PMC Article
Xu-Monette ZY, Zhou J, Young KH
Blood 2018 Jan 4;131(1):68-83. Epub 2017 Nov 8 doi: 10.1182/blood-2017-07-740993. PMID: 29118007Free PMC Article
Martelli M, Ferreri A, Di Rocco A, Ansuinelli M, Johnson PWM
Crit Rev Oncol Hematol 2017 May;113:318-327. Epub 2017 Jan 21 doi: 10.1016/j.critrevonc.2017.01.009. PMID: 28318892

Clinical prediction guides

Noerenberg D, Briest F, Hennch C, Yoshida K, Hablesreiter R, Takeuchi Y, Ueno H, Staiger AM, Ziepert M, Asmar F, Locher BN, Toth E, Weber T, Amini RM, Klapper W, Bouzani M, Poeschel V, Rosenwald A, Held G, Campo E, Ishaque N, Stamatopoulos K, Kanellis G, Anagnostopoulos I, Bullinger L, Goldschmidt N, Zinzani PL, Bödör C, Rosenquist R, Vassilakopoulos TP, Ott G, Ogawa S, Damm F
J Clin Oncol 2024 Feb 1;42(4):452-466. Epub 2023 Dec 6 doi: 10.1200/JCO.23.01053. PMID: 38055913
Camus V, Viailly PJ, Drieux F, Veresezan EL, Sesques P, Haioun C, Durot E, Patey M, Rossi C, Martin L, Rainville V, Bohers E, Ruminy P, Penther D, Kaltenbach S, Bruneau J, Paillassa J, Tournilhac O, Willaume A, Antier C, Lazarovici J, Lévêque E, Decazes P, Becker S, Tonnelet D, Berriolo-Riedinger A, Gaulard P, Tilly H, Molina TJ, Traverse-Glehen A, Jardin F
Blood Adv 2023 Dec 12;7(23):7331-7345. doi: 10.1182/bloodadvances.2023011169. PMID: 37862676Free PMC Article
Xue Y, Gao S, Gou J, Yin T, He H, Wang Y, Zhang Y, Tang X, Wu R
Expert Opin Drug Deliv 2021 Feb;18(2):187-203. Epub 2020 Oct 5 doi: 10.1080/17425247.2021.1825376. PMID: 32954856
Xu-Monette ZY, Zhou J, Young KH
Blood 2018 Jan 4;131(1):68-83. Epub 2017 Nov 8 doi: 10.1182/blood-2017-07-740993. PMID: 29118007Free PMC Article
Bhatt VR, Mourya R, Shrestha R, Armitage JO
Cancer Treat Rev 2015 Jun;41(6):476-85. Epub 2015 Apr 21 doi: 10.1016/j.ctrv.2015.04.006. PMID: 25913715

Recent systematic reviews

Takyar J, Raut M, Borse R, Balakumaran A, Sehgal M
Expert Rev Hematol 2020 Mar;13(3):275-287. Epub 2020 Jan 28 doi: 10.1080/17474086.2020.1716725. PMID: 31951774

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2024
    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Pediatric Aggressive Mature B-Cell Lymphomas, 2024

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...